By now I’m likely in trouble with my high school biology teacher; that is, trying to explain the circulatory system using a plumbing analogy and all. I recall my biology teacher describing a closed system, a circulatory triad of arteries, capillaries, and veins. Something about the arteries carrying blood away from the heart, the arteries connecting to capillaries, the capillaries in turn to veins, and poof, like magic, blood is back in the lungs and heart for another run-through. He might have even addressed the length of this magical circulatory system. As if committing to memory facts like the blood vessels of an average child laid out in one line are 60,000 miles long and an adult’s vessels are closer to 100,000 miles long will make me a better citizen! Well … ok, if you’re an Intervention Radiologist or a Vascular Surgeon that statistic might be helpful to know. With fond memories of high school biology those lessons now seem a bit off, a bit to simplistic.
Personally having Klippel-Trenaunay Syndrome has become a stark lesson that our blood-based fuel-food delivery system is not really a closed system. I see a more exacting, more complex story about feeding, recouping and releasing associated by-products. There’s the releasing of the food and fuel in the blood to the muscles. There’s the proper feeding of the muscular-skeletal system. Notably, some K-T ‘ers will tell you it’s about aberrations that sometimes result in the over-feeding or under-feeding of these components. Others will say it’s about the body’s built-in vacuum cleaner, the lymph vessels and attached nodes that eventually release or dump very toxic material from the body. A better mental picture is a very elaborate and multilevel system of blood vessels and lymph vessels that serve as the transport system for vital human endeavors. In the end, it’s a story about capillaries, veins and lymph vessels and what happens when these individually or collectively are instructed by our chromosomes to develop in aberrant ways.
Capillaries: The Feeding Bed
Capillaries are very thin and fragile; these are only one cell thick. They are so thin that blood cells only pass through them single file. Red blood cells which are inside the capillary release oxygen which then passes through the capillary wall and into surrounding tissues. When the muscle tissue is done burning up its fuel it releases waste, like carbon dioxide, which passes back through the wall and is absorbed by the red blood cells. Here’s something K-Ters instinctively know. When you exercise your body temperature and blood temperature rises and it’s the capillaries that release this excess heat. That’s why your skin is flushed, that is, red in color. It is also why those with K-T who have capillary malformations see the colorful stains on their body as light red at times. Other times the blood in the capillaries carries a higher degree of deoxygenated blood and thus those glorious painted stains turn purplish in color.
Veins: Recycling at its Best
Veins and arteries have some physical similarities. Both have three layers – an outer layer, muscle in the middle, and an inner layer. Veins are thinner and containing less tissue. Veins are weaker. They transport blood at lower pressure. The color of blood in the veins is a deep red color, almost purple. Since the walls of the veins are rather thin, this blood can be visible through the skin at times. The veins job is to receive spent blood from the capillaries and transport this waste-rich blood back to the lungs and heart. Valves are located inside the veins to keep the blood moving in the proper direction. Back flow is not a good idea. The valves are like gates that only allow traffic to move in one direction. These also fight the force of gravity which pulls blood back. This is another dynamic that K-Ters all too often instinctively know about. Vascular malformations of the veins include valves that under develop or never develop, as well as veins that are too large in circumference thus causing leakage at the valve. In my case, the deep veins in my right leg never developed and those in my left leg were so incompetent that the blood fails to flow back to the buttocks and torso. Did you know your butt is an important part of the venous system? Oh ya, like if you sit on it you’re cutting off a significant amount of return flow to your lungs and heart. And you thought the big negative about being lazy was all about not using your leg muscles.
Lymph Vessel Malformations: An Unexpected House Guest
More to come in the next article – suffice it to say for now that Cellulitis is a huge problem for most KTers – if not in the early years, almost universal in our latter years. Having a basic understanding of the lymph system is vital.
When Feeding the Lion is Dangerous
Many KTers experience complications known as hypertrophy or hemitrophy. These folks have very difficult challenges and often seek the most aggressive surgical treatments of our group. I’m working to gather more information on these conditions as well. You will hear about debulking procedures, limb shortening, limb lengthening and amputations. K-T is not an academic matter. It affects our lives in incomprehensible ways. Living with it is the only way to really understand its nuances. As such, I’m reaching out to several KTS kids with experience in these matters and I hope they will write and post here. If they prefer to maintain their privacy, it is more than understandable and then I will do my best to be their voice without betraying their trust and friendship.
… the project continues, but for the balance of today I rest.
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KTS kids ©2011
K-T kids ©2011